Epidemiology of autoimmune liver disease

Abstract

Primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are chronic liver diseases that likely have an autoimmune basis to their pathogenesis. Although significant strides have been made in the clinical management of these conditions, their pathogenesis remains obscure. Understanding of various epidemiological factors may shed light on predisposing or causative factors for these diseases. Most is known about the epidemiology of PBC, with only minimal information on that of PSC and AIH. In this review, the current data on the epidemiology of PBC, AIH and PSC are summarized and suggestions are made for future work in this important area.

Hepatitis B virus is still the most common etiologic factor of liver cirrhosis: Results from a single center in Turkey

“…Unfortunately, considerably less is known about the prevalence of autoimmune diseases in patients with LC. There is a marked female predominance in AIH and PBS but PSC is more common in men [13]. In our study, patients with autoimmune etiologies comprised 7.4% of cases and were predominantly female.…”

Liver enzyme alteration: a guide for clinicians

“…For example, about 60% of cases of elevated hepatic AST levels in Wales can be attributed to ischemic or toxic liver injury, 10 whereas orofecal transmittable hepatitis viruses are the major cause (> 60%) of sporadic acute and fulminant hepatitis in the Far East. 11 The incidence of primary biliary cirrhosis ranges from 1.9-2.2 per 100 000 in Australia to 0.34-0.42 per 100 000 in Asia, 12 and the prevalence of homozygosity for the C282Y mutation in the HFE gene can be found in about 5 per 1000 people of northern European descent 13 but 0.0001 per 100 people of African American ethnicity.…”

Autoimmune liver disease: overlap and outliers

“…PSC is slightly less common than PBC, and is rare in regions with a low prevalence of inflammatory bowel disease. The prevalence of PSC in the USA is estimated as 1-4 cases/100 000 population, 36 but this is likely to be an underestimate.…”

“…38 Like PBC, PSC is considered to be a disease of autoimmunity, and a marked increase in prevalence of HLA antigens B8 and DR3 has been found in patients with PSC. 36 The HLA-DR3, DQ2 heterozygous genotype may be associated with more rapid disease progression. 39 The HLA B8, DR3 haplotype has been associated with a number of autoimmune diseases such as AIH, thyroiditis, celiac disease, and myasthenia gravis.…”